HR-MRI may reveal characteristic vessel wall changes of MMD (shrinkage) rather than atherosclerotic plaque on the stenotic segment.[17,18] Our present study of HR-MRI- and conventional angiography-confirmed ICAS cases indicates that the RNF213 variant is also associated with ICAS, and suggests that ICAS and MMD share common genetic polymorphisms. Here, RNF213 is linked to familial isolated congenital asplenia.